Chapter 17. End-of-life care in Friedreich ataxia
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
Disclaimer / Intended Use / Funding
Disclaimer
The Clinical Management Guidelines for Friedreich ataxia (‘Guidelines’) are protected by copyright owned by the authors who contributed to their development or said authors’ assignees.
These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
Guidelines users must seek out the most recent information that might supersede the diagnostic and treatment recommendations contained within these Guidelines and consider local variations in clinical settings, funding and resources that may impact on the implementation of the recommendations set out in these Guidelines.
The authors of these Guidelines disclaim all liability for the accuracy or completeness of the Guidelines, and disclaim all warranties, express or implied to their incorrect use.
Intended Use
These Guidelines are made available as general information only and do not constitute medical advice. These Guidelines are intended to assist qualified healthcare professionals make informed treatment decisions about the care of individuals with Friedreich ataxia. They are not intended as a sole source of guidance in managing issues related to Friedreich ataxia. Rather, they are designed to assist clinicians by providing an evidence-based framework for decision-making.
These Guidelines are not intended to replace clinical judgment and other approaches to diagnosing and managing problems associated with Friedreich ataxia which may be appropriate in specific circumstances. Ultimately, healthcare professionals must make their own treatment decisions on a case-by-case basis, after consultation with their patients, using their clinical judgment, knowledge and expertise.
Guidelines users must not edit or modify the Guidelines in any way – including removing any branding, acknowledgement, authorship or copyright notice.
Funding
The authors of this document gratefully acknowledge the support of the Friedreich Ataxia Research Alliance (FARA). The views and opinions expressed in the Guidelines are solely those of the authors and do not necessarily reflect the official policy or position of FARA.
17. End-of-life care in Friedreich ataxia
This chapter addresses important issues related to end-of-life care for individuals with Friedreich ataxia, including advance care planning, palliative care and the option of hospice care in the final stages of life. Although relevant to end-of-life care, palliative care can also be useful at earlier stages in a person’s life and advance car planning should be discussed early on so that an individual with Friedreich ataxia can retain control over their treatment and management. In making recommendations for management of end-of-life care, the authors were tasked with answering the following questions:
For individuals with Friedreich ataxia what is the best management for advance care planning? (see 17.1)
For individuals with Friedreich ataxia what is the best management for palliative care? (see 17.2)
For individuals with Friedreich ataxia what is the best management for end-of-life (hospice) care? (see 17.3)
Mary G. Kearney, MD
Neurology Research Fellow, Tallaght University Hospital, Dunlavin, Wicklow, Ireland
Email: marykearney@gmail.com
Lauren Treat, MD
Assistant Professor of Pediatrics, University of Colorado School of Medicine, Aurora, CO, USA
Email: lauren.treat@childrenscolorado.org
Neal J. Weisbrod, MD
Assistant Professor of Neurology, University of Florida, University of Florida, Florida, USA
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11. Bakitas MA, Dionne-Odom JN, Ejem DB, Wells R, Azuero A, Stockdill ML, et al. Effect of an early palliative care telehealth intervention vs usual care on patients with heart failure: the ENABLE CHF-PC randomized clinical trial. JAMA Intern Med. 2020;180(9):1203-13.
12. Sahlen KG, Boman K, Brannstrom M. A cost-effectiveness study of person-centered integrated heart failure and palliative home care: Based on a randomized controlled trial. Palliat Med. 2016;30(3):296-302.
13. Dionne-Odom JN, Ejem DB, Wells R, Azuero A, Stockdill ML, Keebler K, et al. Effects of a telehealth early palliative care intervention for family caregivers of persons with advanced heart failure: The ENABLE CHF-PC randomized clinical trial. JAMA Netw Open. 2020;3(4):e202583.
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16. De Michele G, Perrone F, Filla A, Mirante E, Giordano M, De Placido S, et al. Age of onset, sex, and cardiomyopathy as predictors of disability and survival in Friedreich’s disease: a retrospective study on 119 patients. Neurology. 1996;47(5):1260-4.
17. Lah S, Wilson EL, Beesley S, Sagy I, Orme J, Novack V, et al. Association of hospice utilization and publicly reported outcomes following hospitalization for pneumonia or heart failure: a retrospective cohort study. BMC Health Serv Res. 2018;18(1):12.
18. Yim CK, Barron Y, Moore S, Murtaugh C, Lala A, Aldridge M, et al. Hospice enrollment in patients with advanced heart failure decreases acute medical service utilization. Circ Heart Fail. 2017;10(3):e003335.
These Guidelines are systematically developed evidence statements incorporating data from a comprehensive literature review of the most recent studies available (up to the Guidelines submission date) and reviewed according to the Grading of Recommendations, Assessment Development and Evaluations (GRADE) framework © The Grade Working Group.
This chapter of the Clinical Management Guidelines for Friedreich Ataxia and the recommendations and best practice statements contained herein were endorsed by the authors and the Friedreich Ataxia Guidelines Panel in 2022.
It is our expectation that going forward individual topics can be updated in real-time in response to new evidence versus a re-evaluation and update of all topics simultaneously.